Third nerve palsy as an initial presentation of neurofibromatosis 2

Deepanjali Arya; Sima Das

doi:10.4103/ijo.IJO_332_20

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Year : 2021 | Volume : 1 | Issue : 1 | Page : 79

Third nerve palsy as an initial presentation of neurofibromatosis 2

Deepanjali Arya, Sima Das
Oculoplasty and Ocular Oncology Services, Dr Shroff's Charity Eye Hospital, New Delhi, India

Date of Submission	20-Feb-2020
Date of Acceptance	19-May-2020
Date of Web Publication	31-Dec-2020

Correspondence Address:
Dr. Sima Das
128 Ankur Apartments, & I P Extension, Patparganj, New Delhi
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_332_20

Abstract

Keywords: Neurofibromatosis type 2, ptosis, third nerve palsy

How to cite this article:
Arya D, Das S. Third nerve palsy as an initial presentation of neurofibromatosis 2. Indian J Ophthalmol Case Rep 2021;1:79

How to cite this URL:
Arya D, Das S. Third nerve palsy as an initial presentation of neurofibromatosis 2. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Mar 3];1:79. Available from: https://www.ijoreports.in/text.asp?2021/1/1/79/305523

A 14-year-old boy presented with complaints of progressive drooping of the left upper eyelid for the past 4 years. Best-corrected visual acuity (BCVA) was 20/20 in both eyes. The right eye anterior segment, fundus, and extraocular movements were normal. The left eye extraocular movements were restricted in elevation, depression, and adduction with moderate left upper lid ptosis [Figure 1]. Left eye pupil was dilated, non-reacting. The rest of the cranial nerve examinations were normal. Systemic examination revealed multiple café-au-lait spots on the back and thighs.

Figure 1: (a) Clinical photograph at initial presentation showing moderate ptosis and left exotropia and mid dilated pupil (b) Progression at 3 years causing total external ophthalmoplegia and severe ptosis (c) MRI brain (coronal scan) showing bilateral intracranial schwannoma, larger on the left side (d)Appearance at 3 year follow up showing severe ptosis and total ophthalmoplegia left eye

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A contrast magnetic resonance imaging (MRI) showed multiple variable-sized enhancing lesions along the course of 3^rd, 5^th, 7^th, and 8^th nerves bilaterally consistent with trigeminal and vestibule-cochlear schwannomas. Multiple small, schwannoma were seen along the spinal cord surface along with an enhancing lesion at the cervico-medullary junction suggestive of intramedullary ependymoma or astrocytoma. The patient was diagnosed as left third nerve palsy with pupil involvement secondary to neurofibromatosis 2 (NF2) and was referred to a neurosurgeon where monitoring for progression was advised due to high surgical morbidity. At 3-years follow-up, total external ophthalmoplegia and mildly reduced left corneal sensations were noted with no systemic symptoms. Repeat MRI showed a slight increase in spinal tumor size and stable cranial lesions. On neurosurgery review, follow-up was advised owing to stable lesions on imaging.

Discussion

Third nerve palsy involving pupil is a rare initial presentation of NF2. Ocular symptoms are a more common presenting feature of NF2 in the younger age group than extraocular symptoms.^[1],[2] Pupillary involvement indicates a compressive etiology which were the intracranial schwannomas along the course of the third nerve in our patient. A similar presentation by Tevaraj et al. was postulated to be due to a lesion around the anterior cavernous sinus.^[3]

The clinical presentation of NF2 is variable. Third nerve palsy can be the initial presentation of NF2 in a younger age group.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Perry A, Giannini C, Raghavan R, Scheithauer BW, Banerjee R, Margraf L, et al. Aggressive phenotypic and genotypic features in pediatric and NF2-associated meningiomas: A clinicopathologic study of 53 cases. J Neuropathol Exp Neurol 2001;60:994-1003.
2.	Ragge NK, Baser ME, Riccardi VM, Falk RE. The ocular presentation of neurofibromatosis 2. Eye 1997;11:12-8.
3.	Tevaraj JM, Li Min ET, Mohd Noor RA, Yaakub A, Wan Hitam WH. Neurofibromatosis type 2 presenting with oculomotor ophthalmoplegia and distal myopathy. Ophthalmol Med 2016:1701509.